Hostname: page-component-586b7cd67f-r5fsc Total loading time: 0 Render date: 2024-11-23T14:57:16.607Z Has data issue: false hasContentIssue false
  • English
  • Français

Changing attitudes to the management of hypoplastic left heart syndrome: a European perspective

Published online by Cambridge University Press:  13 December 2011

Bari Murtuza  and
Martin J. Elliott
Bari Murtuza
Affiliation:
Department of Paediatric Cardiothoracic Surgery, The Great Ormond Street Hospital for Children National Health Service Trust, London, United Kingdom
Martin J. Elliott*
Affiliation:
Department of Paediatric Cardiothoracic Surgery, The Great Ormond Street Hospital for Children National Health Service Trust, London, United Kingdom
*
Correspondence to: Prof. M. J. Elliott, Department of Cardiothoracic Surgery, The Great Ormond Street Hospital for Children, London WC1N 3JH, United Kingdom. Tel: +44 (0) 207 829 8853; Fax: +44 (0) 207 813 8262; E-mail: [email protected]
Get access Rights & Permissions [Opens in a new window]

Abstract

Background and aims

Several years ago, one of us described the difference in attitude to patients with hypoplastic left heart syndrome in the United States of America and Europe as similar to that between Mars and Venus. Uncertainty remains with regard to the long-term prognosis for patients with hypoplastic left heart syndrome. This prognosis may be considered in terms of survival, functional performance, including exercise capacity and neurodevelopment, as well as psychosocial effects on the patient, family, and siblings. Counselling parents where either an antenatal or postnatal diagnosis of hypoplastic left heart syndrome has been made requires practitioners to give information on these prognostic aspects. We wanted to see how attitudes among European surgeons have changed over the last few years.

Methods

We performed a review of recent European data for hypoplastic left heart syndrome and conducted a survey among surgeons in major European centres to ascertain key aspects of their attitudes to the management of patients with hypoplastic left heart syndrome and how they counsel parents.

Results and conclusions

As of January, 2011, 2392 citations in the PubMed database were available for the search string “hypoplastic left heart”. The majority of these were from the centres from the United States of America and Europe. The European Association for Cardio-Thoracic Surgery Congenital Heart Surgery Database shows an annual increase in the number of Norwood (Stage I) operations for hypoplastic left heart syndrome from 2003 to 2009, with a corresponding reduction in mortality. European rates of antenatal detection vary widely between centres, as do the rates of termination for a prenatal diagnosis of hypoplastic left heart syndrome. We observed a wide variation in the estimates of surgeons for survival and quality of life for surgical palliation of hypoplastic left heart syndrome, as well as in their estimates for actual rates of termination of pregnancy in their centres. Further, there was marked inconsistency in the information given to parents as part of the process of counselling. These issues remain to be resolved if parents are to make a fully informed decision for their child.

Keywords

Norwoodhypoplastic left heart syndromehypoplasia of the left heartsingle-ventricle repairpregnancy terminationcomfort caresurgical palliationfoetal diagnosisantenatal diagnosisEuropeancounselling
Type
Original Article
Information
Cardiology in the Young , Volume 21 , Issue S2: A Holistic Approach to Hypoplastic Left Heart Syndrome and other evolving challenges in Pediatric and Congenital Cardiac Disease , 13 December 2011 , pp. 148 - 158
Copyright
Copyright © Cambridge University Press 2011

Access options

Get access to the full version of this content by using one of the access options below. (Log in options will check for institutional or personal access. Content may require purchase if you do not have access.)

References

1.Elliott, MJ. A European perspective on the management of hypoplastic left heart syndrome. Cardiol Young 2004; 14 Suppl 1: 41–46.CrossRefGoogle ScholarPubMed
2.Kon, AA, Ackerson, L, Lo, B. How pediatricians counsel parents when no “best-choice” management exists: lessons to be learned from hypoplastic left heart syndrome. Arch Pediatr Adolesc Med 2004; 158: 436–441.CrossRefGoogle ScholarPubMed
3.Moons, P, Bovijn, L, Budts, W, Belmans, A, Gewillig, M. Temporal trends in survival to adulthood among patients born with congenital heart disease from 1970 to 1992 in Belgium. Circulation 2010; 122: 2264–2272.CrossRefGoogle ScholarPubMed
4.Furck, AK, Uebing, A, Hansen, JH, et al. Outcome of the Norwood operation in patients with hypoplastic left heart syndrome: a 12-year single-center survey. J Thorac Cardiovasc Surg 2010; 139(2): 359–365CrossRefGoogle ScholarPubMed
5.Sano, S, Huang, SC, Kasahara, S, Yoshizumi, K, Kotani, Y, Ishino, K. Risk factors for mortality after the Norwood procedure using right ventricle to pulmonary artery shunt. Ann Thorac Surg 2009; 87: 178–185; discussion 85-6.CrossRefGoogle ScholarPubMed
6.Murtuza, B, Wall, D, Reinhardt, Z, et al. The importance of blood lactate clearance as a predictor of early mortality following the modified Norwood procedure. Eur J Cardiothorac Surg 2011; 40(5): 1207–1214.Google ScholarPubMed
7.Ohye, RG, Sleeper, LA, Mahony, L, et al. Comparison of shunt types in the Norwood procedure for single-ventricle lesions. N Engl J Med 2010; 362: 1980–1992.CrossRefGoogle ScholarPubMed
8.Galantowicz, M, Cheatham, JP, Phillips, A, et al. Hybrid approach for hypoplastic left heart syndrome: intermediate results after the learning curve. Ann Thorac Surg 2008; 85: 2063–2070; discussion 70-1.CrossRefGoogle ScholarPubMed
9.Marshall, AC, Levine, J, Morash, D, et al. Results of in utero atrial septoplasty in fetuses with hypoplastic left heart syndrome. Prenat Diagn 2008; 28: 1023–1028.CrossRefGoogle ScholarPubMed
10.Karamlou, T, Diggs, BS, Ungerleider, RM, Welke, KF. Evolution of treatment options and outcomes for hypoplastic left heart syndrome over an 18-year period. J Thorac Cardiovasc Surg 2010; 139: 119–126; discussion 26-7.CrossRefGoogle ScholarPubMed
11.Hirsch, JC, Gurney, JG, Donohue, JE, Gebremariam, A, Bove, EL, Ohye, RG. Hospital mortality for Norwood and arterial switch operations as a function of institutional volume. Pediatr Cardiol 2008; 29: 713–717.CrossRefGoogle ScholarPubMed
12.Welke, KF, O'Brien, SM, Peterson, ED, Ungerleider, RM, Jacobs, ML, Jacobs, JP. The complex relationship between pediatric cardiac surgical case volumes and mortality rates in a national clinical database. J Thorac Cardiovasc Surg 2009; 137: 1133–1140.CrossRefGoogle Scholar
13.NHS Specialised Services. Safe and Sustainable: Childrens’ Congenital Heart Services in England. NHS Specialised Services, London, UK, 2011.Google Scholar
14.Gaynor, JW, Gerdes, M, Nord, AS, et al. Is cardiac diagnosis a predictor of neurodevelopmental outcome after cardiac surgery in infancy? J Thorac Cardiovasc Surg 2010; 140: 1230–1237.CrossRefGoogle ScholarPubMed
15.Licht, DJ, Shera, DM, Clancy, RR, et al. Brain maturation is delayed in infants with complex congenital heart defects. J Thorac Cardiovasc Surg 2009; 137: 529–536; discussion 36-7.CrossRefGoogle ScholarPubMed
16.Brosig, CL, Mussatto, KA, Kuhn, EM, Tweddell, JS. Psychosocial outcomes for preschool children and families after surgery for complex congenital heart disease. Pediatr Cardiol 2007; 28: 255–262.CrossRefGoogle ScholarPubMed
17.Creighton, DE, Robertson, CM, Sauve, RS, et al. Neurocognitive, functional, and health outcomes at 5 years of age for children after complex cardiac surgery at 6 weeks of age or younger. Pediatrics 2007; 120: e478–e486.CrossRefGoogle ScholarPubMed
18.Srinivasan, C, Jaquiss, RD, Morrow, WR, et al. Impact of staged palliation on somatic growth in patients with hypoplastic left heart syndrome. Congenit Heart Dis 2010; 5: 546–551.CrossRefGoogle ScholarPubMed
19.Moller, P, Weitz, M, Jensen, KO, et al. Exercise capacity of a contemporary cohort of children with hypoplastic left heart syndrome after staged palliation. Eur J Cardiothorac Surg 2009; 36: 980–985.CrossRefGoogle ScholarPubMed
20.Mellander, M, Berntsson, L, Nilsson, B. Quality of life in children with hypoplastic left heart syndrome. Acta Paediatr 2007; 96: 53–57.CrossRefGoogle ScholarPubMed
21.Rempel, GR, Harrison, MJ. Safeguarding precarious survival: parenting children who have life-threatening heart disease. Qual Health Res 2007; 17: 824–837.CrossRefGoogle ScholarPubMed
22.Mahle, WT, Clancy, RR, McGaurn, SP, Goin, JE, Clark, BJ. Impact of prenatal diagnosis on survival and early neurologic morbidity in neonates with the hypoplastic left heart syndrome. Pediatrics 2001; 107: 1277–1282.CrossRefGoogle ScholarPubMed
23.Tworetzky, W, McElhinney, DB, Reddy, VM, Brook, MM, Hanley, FL, Silverman, NH. Improved surgical outcome after fetal diagnosis of hypoplastic left heart syndrome. Circulation 2001; 103: 1269–1273.CrossRefGoogle ScholarPubMed
24.McBrien, A, Sands, A, Craig, B, Dornan, J, Casey, F. Major congenital heart disease: antenatal detection, patient characteristics and outcomes. J Matern Fetal Neonatal Med 2009; 22: 101–105.CrossRefGoogle Scholar
25.Atz, AM, Travison, TG, Williams, IA, et al. Prenatal diagnosis and risk factors for preoperative death in neonates with single right ventricle and systemic outflow obstruction: screening data from the Pediatric Heart Network Single Ventricle Reconstruction Trial(*). J Thorac Cardiovasc Surg 2010; 140: 1245–1250.CrossRefGoogle ScholarPubMed
26.Galindo, A, Nieto, O, Villagra, S, Graneras, A, Herraiz, I, Mendoza, A. Hypoplastic left heart syndrome diagnosed in fetal life: associated findings, pregnancy outcome and results of palliative surgery. Ultrasound Obstet Gynecol 2009; 33: 560–566.CrossRefGoogle ScholarPubMed
27.Rasiah, SV, Ewer, AK, Miller, P, et al. Antenatal perspective of hypoplastic left heart syndrome: 5 years on. Arch Dis Child Fetal Neonatal Ed 2008; 93: F192–F197.CrossRefGoogle Scholar
28.Garne, E, Stoll, C, Clementi, M. Evaluation of prenatal diagnosis of congenital heart diseases by ultrasound: experience from 20 European registries. Ultrasound Obstet Gynecol 2001; 17: 386–391.CrossRefGoogle ScholarPubMed
29.Garne, E, Khoshnood, B, Loane, M, Boyd, P, Dolk, H. Termination of pregnancy for fetal anomaly after 23 weeks of gestation: a European register-based study. BJOG 2010; 117: 660–666.CrossRefGoogle ScholarPubMed
30.Menahem, S, Grimwade, J. Pregnancy termination following prenatal diagnosis of serious heart disease in the fetus. Early Hum Dev 2003; 73: 71–78.CrossRefGoogle ScholarPubMed
31.Prsa, M, Holly, CD, Carnevale, FA, Justino, H, Rohlicek, CV. Attitudes and practices of cardiologists and surgeons who manage HLHS. Pediatrics 2010; 125: e625–e630.CrossRefGoogle ScholarPubMed
32.Corrow, C, Lapuk, S, Mazzarella, K, Sable, A, Leopold, H, Eisenfeld, L. Hypoplastic left heart syndrome: factors influencing therapeutic choice. Conn Med 2001; 65: 195–203.Google ScholarPubMed
33.Osiovich, H, Phillipos, E, Byrne, P, Robertson, M. Hypoplastic left heart syndrome: “to treat or not to treat”. J Perinatol 2000; 20: 363–365.CrossRefGoogle Scholar
34.Renella, P, Chang, RK, Ferry, DA, Bart, RD, Sklansky, MS. Hypoplastic left heart syndrome: attitudes among pediatric residents and nurses towards fetal and neonatal management. Prenat Diagn 2007; 27: 1045–1055.CrossRefGoogle ScholarPubMed
35.Vandvik, IH, Forde, R. Ethical issues in parental decision-making. An interview study of mothers of children with hypoplastic left heart syndrome. Acta Paediatr 2000; 89: 1129–1133.CrossRefGoogle ScholarPubMed
36.Williams, DL, Gelijns, AC, Moskowitz, AJ, et al. Hypoplastic left heart syndrome: valuing the survival. J Thorac Cardiovasc Surg 2000; 119: 720–731.CrossRefGoogle ScholarPubMed
37.World Health Organisation. Countdown to 2015: Maternal, Newborn and Child Survival. World Health Organisation, Geneva, Switzerland, 2010.Google Scholar